journal of Neuro-Ophthalmology 14(2): 91-94, 1994. 1994 Raven Press, Ltd., New York Spontaneous Remission of Papilledema and Sixth Nerve Palsy in Acute Lymphoblastic Leukemia Gil I. Wolfe, M.D., Steven L. Galetta, M.D., and Joan E. Mollman, M.D. Spontaneous regression of hematologic malignancies is not uncommon and occurs in a wide variety of lympho- mas and leukemias. In contrast, spontaneous remission of neurologic symptoms produced by these tumors is exceedingly rare. We report a patient with central ner- vous system acute lymphoblastic leukemia who experi- enced at least one spontaneous remission of papillede- ma and sixth nerve palsy. This represents, to our knowl- edge, the first case of spontaneous remission of neuro- ophthalmologic signs in a patient with acute leukemia. We conclude that meningeal leukemia may have a pro- tracted course, and that spontaneous remission of neuro-ophthalmologic findings should not be so readily ascribed to a benign process in a patient with preexisting leukemia. Key Words: Spontaneous remission—Acute lympho- blastic leukemia—Leptomeningeal metastasis— Papilledema—Sixth nerve palsy. From the Department of Neurology, University of Pennsyl- vania School of Medicine, Philadelphia, Pennsylvania, U.S.A. Address correspondence and reprint requests to Dr. Steven L. Galetta, Department of Neurology, Hospital of the Univer- sity of Pennsylvania, 3400 Spruce St., Philadelphia, PA 19104. The spontaneous regression of cancer refers to the partial or complete disappearance of malignant tumor in the setting of no treatment or treatment judged inadequate to explain the observed remis- sion (1,2). Spontaneous regression has been re- ported in a wide variety of cancers, including solid tumors and hematologic malignancies (1,3-5). The duration of spontaneous regression in lymphoma can be prolonged and even permanent (3,6-9). In contrast, spontaneous regression of acute leuke- mias tends to be short-lived (3,10-14). Documented regression of hematologic malig- nancies that involve the central nervous system (CNS) is exceedingly rare. Rubin and colleagues (15) reported a complete spontaneous regression of primary CNS lymphoma, which lasted 13 months. Prolonged survival has been described in cases of meningeal lymphoma (16) and neurolym- phomatosis (17), where the diagnosis was delayed from months to years. Spontaneous remission of neurologic symptoms produced by these tumors is equally rare. Galetta and colleagues (18) recently reported the spontaneous resolution of a third nerve palsy in a patient subsequently found to have meningeal lymphoma. On repeat magnetic resonance imaging (MRI), abnormal enlargement and enhancement of the patient's third nerve also resolved. To our knowledge, spontaneous remis- sion of neurologic symptoms has never been ob- served in meningeal leukemia. We report a patient with CNS acute lymphoblastic leukemia (ALL) who had relapsing and remitting episodes of dip- lopia and papilledema over a 3-year period with- out definitive treatment. CASE REPORT The patient presented in August 1984 at age 16 with leg pain, a 5-lb weight loss, and fatigue. Phys- 91